Infradiaphragmatic Total Anomalous Pulmonary Venous Return

Transcription

1 Infradiaphragmatic Total Anomalous Pulmonary Venous Return Report of a New and Correctable Variant J. Kent Trinkle, M.D., Gordon K. Danielson, M.D., Jacqueline A. Noonan, M.D., and Charles Stephens, M.D. T otal anomalous pulmonary venous return (TAPVR) is a rare cardiac anomaly in which all pulmonary venous blood returns to the right side of the heart. The pulmonary veins combine to form a common retrocardiac venous pool-the transverse anomalous vein-which drains into the right atrium either directly, via the coronary sinus, or via one of the systemic veins. A patent foramen ovale or atrial septa1 defect, which is always present, allows blood to reach the left atrium. There are three types of TAPVR: (1) supracardiac-pulmonary venous blood returns via a vertical vein which joins the superior vena cava or the left innominate vein; (2) cardiac-pulmonary venous blood returns to the right atrium directly or to the coronary sinus; and (3) infracardiac-pulmonary venous blood from the transverse vein returns via a descending vertical anomalous vein which passes through the diaphragmatic hiatus and joins the inferior vena cava or one of its branches. This report describes a variant of infracardiac TAPVR which has not been previously reported. J. P., a four-week-old white female, was the product of a 38-week pregnancy. She was delivered by emergency cesarean section due to an abruptio placenta; birth weight was 2,528 gm. At birth she required vigorous resuscitation and meconium-stained mucus was aspirated from the lungs. CNS depression was present for several days. Chest x-ray revealed a ground glass appearance to the lungs. No cardiac murmurs were heard, but the electrocardiogram indicated right ventricular hypertrophy. The initial diagnosis was either hyaline membrane disease or aspiration pneumonitis. She was treated with oxygen and digitalis. Although her general condition improved, the tachypnea and intermittent cyanosis persisted. Upon admission to the University of Kentucky Medical Center, J. P. weighed 2,980 gm. and was active, but she had tachypnea at rest. Pulse was 162 per minute and respiration 50 per minute. Pulses were good in all extremities. She was dusky at rest, but she became cyanotic and had intercostal retractions From the Departments of Surgery, Pediatrics, and Pathology, University of Kentucky Medical Center, Lexington, Ky. Accepted for publication Sept. 6, VOL. 5, NO. 1, JAN.,

2 TRINKLE, DANIELSON, NOONAN, AND STEPHENS with crying. There was a grade 2 of 4 systolic ejection murmur along the second intercostal space, and the pulmonic second sound was increased. There were no thrills or peripheral edema, and the liver and spleen were normal. Chest x-ray indicated that the heart was within normal limits of size, and that there was increased pulmonary blood flow (Fig. 1). Hemoglobin was 12.8 gm.; hematocrit, 42%; urinalysis, normal; sweat chloride, 15 meq./liter; and stool trypsin was positive. J. P. was initially treated with sodium nafcillin, Ampicillin, elixir of digoxin, and vitamins. Although primary lung disease was the clinical diagnosis, congenital heart disease was considered. Cardiac catheterization revealed the pulmonary and systemic arterial pressures to be equal. A patent ductus arteriosus was present with reversed flow of most of the contrast media from the pulmonary artery into both the aortic arch and the descending aorta. Femoral artery oxygen saturation was 55%. A vertical anomalous pulmonary vein which emptied below the diaphragm was poorly visualized by cineangiography. A diagnosis was made of infracardiac TAPVR with a patent ductus arteriosus. Operation was undertaken the following day. A median sternotomy was made, and the vena cavae and ascending aorta were cannulated. The patent ductus arteriosus was ligated and cardiopulmonary bypass started. The heart was elevated, and a small transverse vein was located through a transverse incision in the posterior pericardium. This vessel joined a vertical anomalous vein which passed toward the esophageal hiatus. Both the transverse and vertical veins were unusually small-measuring not more than 3 mm. in diameter. An open anastomosis was performed between the transverse vein and the posterior left atrium. The vertical anomalous vein was ligated. A 1.0 cm. atrial septa1 defect was not repaired. Bypass was discontinued and the cannulas were removed. The patient initially maintained a normal arterial pressure, but after 10 minutes cardiac action deteriorated. Despite vigorous resuscitative attempts, the patient died on the operating table. Autopsy revealed that the transverse and vertical veins seen at operation drained only the upper lobe of each lung. The vertical vein was connected at the diaphragmatic hiatus with a second similar, but larger, system which drained the lower lobes. Since the inferior system was posterior to the pericardium, it had not FIG. 1. Note normal cardiac size and increased uascular markings. 56 THE ANNALS OF THORACIC SURGERY

3 CASE REPORT: Anom,alous Venous Return ivc I'L FIG. 2. SVC, Superior vena cava; IVC, inferior vena cava; RA, right atrium; Ai, anastomotic incision; STAPV, superior transverse anomalous pulmonary vein; L, point of ligation; SVAPV, superior vertical anomalous pulmonary vein; ITAPV, inferior transverse anomalous pulmonary vein; IVAPV, inferior vertical anomalous pulmonary vein. been visualized at operation and the TAPVR had been only partially corrected. The inferior transverse vein was approximately three times the diameter of the superior transverse vein and the inferior vertical anomalous vein was connected with the inferior vena cava 1 cm. below the diaphragm (Fig. 2). No anomalies were found other than the atrial septa1 defect, the patent ductus arteriosus, and TAPVR. DISCUSSION In the 2.5 mm. human embryo the laryngeal-tracheal ridge forms on the ventral side of the foregut; at 4 mm. paired lung buds are visible. The primordial pulmonary venous plexus has cranial and caudal anastomoses with the vagus venous plexus which in turn communicates inferiorly with the splanchnic plexus and superiorly with the anterior cardinal veins. A dorsal evagination occurs in the sinu-atrial region (primordial left atrium) and extends toward the mesocardium where it normally joins the pulmonary venous plexus. Pulmonary venous drainage is then via the primordial left atrium and the communications (via the vagal plexus) with the splanchnic plexus and the cardinal veins obliterate [2, 5, In TAPVR there is a failure of contact between the dorsal evagination of the primordial left atrium and the pulmonary venous plexus (transverse vein). This causes a persistence of the communication between the pulmonary venous plexus and either the splanchnic or cardinal veins via a remnant of the vagal plexus (vertical vein). The cardiac-type TAPVR would occur if the left anterior cardinal vein maintained its connection with the future coronary sinus or with the right atrial portion of the sinus venosus. Supracardiac TAPVR via VOL. 5, NO. 1, JAN.,

4 TRINKLE, DANIELSON, NOONAN, AND STEPHENS the left innominate vein occurs when the anterior cardinal vein fails to obliterate and maintains its connection with the left innominate vein. Communication with the superior vena cava can occur with persistent communication of the right bronchial vein with the azygos vein. Infracardiac TAPVR will occur with a persistent communication between the pulmonary and the vagal venous plexus draining inferiorly into the splanchnic plexus. The splanchnic plexus is the primordia of a portion of the inferior vena cava, the portal vein, the ductus venosus, the hepatic veins, and the left gastric vein. Infracardiac TAPVR has been reported with drainage of the vertical anomalous vein into each of these veins [4]. TAPVR was first described by Wilson in 1798 [4] and by Friedlosky in 1868 [3]. Abbott [ll found only 4 cases of TAPVR in 1,000 autopsied patients with congenital heart disease; DuShane [8] lists 8 cases of TAPVR in a clinical series of 506 patients with congenital heart disease. In 1942 Brody [31 reported 37 autopsied cases from the literature and described the various patterns. Muller [9] performed the first partial surgical correction of TAPVR in by a closed anastomosis of the posterior wall of the left atrium to the transverse anomalous venous trunk. With improvement in diagnostic techniques and perfection of cardiopulmonary bypass in infants, open anastomosis and total correction has gained popularity. Although the specific operative technique varies according to the pattern of the anomaly, the basic repair is an anastomosis between the transverse anomalous pulmonary venous trunk and the posterior wall of the left atrium, ligation of the systemic venous connection, and repair of the atrial septa1 defect. Infracardiac return is the least common type of TAPVR. Darling and Rothney [7] in 1957 collected 80 autopsy cases of TAPVR from the literature and found only 10 (13%) of the infracardiac type. They also found that TAPVR of all types constituted only 2% of 800 autopsy cases of congenital heart disease at the Boston Children s Hospital. Burrows and Edwards [4] in 1959 collected 188 clinical and autopsy cases of TAPVR from the literature and found 28 patients with anomalous drainage below the diaphragm; they were divided as follows: portal vein, 17; ductus venosus, 6; inferior vena cava, 4; and hepatic vein, 1. They also pointed out that the untreated survival time was shorter in patients with a long anomalous drainage route. The routes which had the highest untreated survival time included drainage into the right atrium, the coronary sinus, or the superior vena cava. A route of intermediate length would be to the left innominate vein, and a long route would include any drainage below the diaphragm. Only 1 out of 28 patients with infracardiac TAPVR survived through adolescence, and 74% of those not operated on were dead at the end of one year [4]. Cooley and associates [6] recently re- 58 THE ANNALS OF THORACIC SURGERY

5 CASE REPORT: Anonz,alous Venous Return ported a 39% mortality rate in 62 operated patients with TAPVR: supracardiac, 35; cardiac, 20; infracardiac, 3; and mixed, 4. Of the 3 infracardiac cases there was only one long-term survivor. One had pulmonary venous drainage into the inferior vena cava and 2 into the portal vein. Due to the short survival time of infants with TAPVR below the diaphragm, immediate operation was performed. The transverse and vertical anomalous veins were quite small, and it was erroneously assumed that the small size of the vessels reflected decreased pulmonary blood flow due to high pulmonary vascular resistance and the known reversed shunt through the patent ductus arteriosus. Since the anomalous pulmonary venous system lies dorsal to the pericardium, the entire pattern of drainage is not visible without an anatomical dissection. The anastomosis to the left atrium was completed with the superior transverse vein which at autopsy proved to drain only the upper lobe of each lung. The inferior transverse vein was approximately three times the diameter of the superior transverse vein and would have been technically easier to anastomose to the posterior wall of the left atrium. SUMMARY The case history of a seriously ill four-week-old female infant with infracardiac TAPVR is presented. A previously unreported anatomical variant was present in which the upper lobe pulmonary veins joined to form a small superior transverse vein and the lower lobe pulmonary veins joined to form a larger inferior transverse vein. The inferior transverse vein drained below the diaphragm into the inferior vena cava. The possibility of this variant should be considered when a small transverse vein is encountered during the repair of infracardiac TAPVR. REFERENCES 1. Abbott, M. E. Atlas of Congenital Heart Disease. New York: The American Heart Association, Auer, J. The development of the human pulmonary vein and its major variations. Anat. Rec. 101:581, Brody, H. Drainage of the pulmonary veins into the right side of the heart. Arch. Path. 33:221, Burroughs, J. T., and Edwards, J. E. Total anomalous pulmonary venous connection. Amer. Heart J. 59:913, Butler, H. Some derivatives of the foregut venous plexus of the albino rat: With reference to man. J. Annt. 86:95, Cooley, D. A., Hallman, G. L., and Leachman, R. D. Total anomalous pulmonary venous drainage. J. Thorac. Cardiov. Surg. 51:88, Darling, R. C., Rothney, W. B., and Craig, J. M. Total pulmonary venous drainage into the right side of the heart. Lab. Invest. 6:44, DuShane, J. W. Total anomalous pulmonary venous connections: Clinical aspects. Proc. Mayo Clin. 31:167, VOL. 5, NO. I, JAN.,

6 TRINKLE, DANIELSON, NOONAN, AND STEPHENS 9. Muller, W. H., Jr. The surgical treatment of transposition of the pulmonary veins. Ann. Surp. 154:683, Mustaid, W. T. Anogalous pulmonary venous drainage. Heart Bull. 13:8, ~~ ~ ~. 11. Shaner, R. F. The development of the bronchial veins, with special reference to anomalies of the pulmonary veins. Anat. Rec. 140: 156, Woodward, G. M., Vince, D. J., and Ashmore, P. G. Total anomalous pulmonary venous return to the portal vein. J. Thorac. Cardiov. Surg. 45:662, THE ANNALS OF THORACIC SURGERY